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Tam Metin / Full Text Q4 SCI-Expanded WOS:000668244900016 PMID: 33315343 The aim of this study is to assess the efficacy and safety of ruxolitinib in patients with myelofibrosis. Materials and methods: From 15 centers, 176 patients (53.4% male, 46.6% female) were retrospectively evaluated. Results: The median age at ruxolitinib initiation was 62 (28–87) and 100 (56.8%) of all were diagnosed as PMF. Constitutional symptoms were observed in 84.7%. The median initiation dose of ruxolitinib was 30 mg (10–40). Dose change was made in 69 (39.2%) patients. Forty seven (35.6%) and 20 (15.2%) of 132 patients had hematological and nonhematological adverse events, respectively. The mean spleen sizes before and after ruxolitinib treatment were 219.67 ± 46.79 mm versus 199.49 ± 40.95 mm, respectively (p < 0.001). There was no correlation between baseline features and subsequent spleen response. Overall survival at 1-year was 89.5% and the median follow up was 10 (1–55) months. We could not show any relationship between survival and reduction in spleen size (p = 0.73). Conclusion: We found ruxolitinib to be safe, well tolerated, and effective in real-life clinical practice in Turkey. Ruxolitinib dose titration can provide better responses in terms of not only clinical benefit but also for long term of ruxolitinib treatment

Purpose Febrile neutropenia (FN) is a hematological emergency. It is challenging and confusing for the clinicians to make the decision of the febrile neutropenic patients under chemotherapy to be monitored at intensive care unit (ICU). The aim of this study was to define the factors supporting decision-making for the critical patients with febrile neutropenia. Methods The data of 60 patients, who were taken to the ICU while they were under treatment in the Hematology Clinic with a diagnosis of febrile neutropenia, were analyzed retrospectively, in order to identify clinically useful prognostic parameters. Results The ICU mortality rate was 80%. Mortality was significantly associated with higher sequential organ failure assessment score (SOFA), quick sequential organ failure assessment score (qSOFA), and hematological SOFA (SOFAhem) scores on admission. All cases having SOFA score 10 and above and qSOFA score 2 and above died. In multivariate analysis, qSOFA score was found to be statistically significant in predicting mortality in regard to ICU admission (p = 0.004). Conclusion Mortality of febrile neutropenic patients admitted to ICU is high. It would be appropriate to determine the extent of organ dysfunction instead of underlying disease, for making the decision of ICU admission. It should be noticed that the risk mortality is high for the FN cases with SOFA score 10 or above, qSOFA score 2 or above, and in need of mechanical ventilation and positive inotropic support; hence, early intervention is recommended. In our study, the most significant parameter in predicting ICU mortality was found to be qSOFA.

Objective: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder that can be especially seen in children and young adults. The clinical presentation of patients with LCH varies according to the sites of involvement. In about half of patients, the disease is limited to a single organ system and bone involvement is very common. In this study, it was aimed to retrospectively evaluation the patients diagnosed with LCH who were followed up and treated in our clinic. Methodology: The data of patients over the age of 18 years who were followed up and treated in Bozyaka Training and Research Hospital Hematology Clinic between 2015-2021 were scanned retrospectively from the hospital system. Results: Data of 6 patients were obtained. The mean age of the patients was 33.6. There was no difference between the genders. Pain was the reason for admission in 4 patients and was the most common symptom. While the most frequently involved system was the skeletal system with 5 patients, lung involvement was seen in 2 patients. Vinblastine and prednisolone combination therapy was given to 1 patient, who developed steroid-related avascular necrosis. One patient who was planned for combination treatment Conclusion: LCH is a rare disease especially seen in children and young adults. It can involve the skeletal system, lungs, and other organs. The prognosis is often good with excision of the lesion or systemic treatment.

We aimed to analyze 10-year experience of WAIHA patients at a single referral center in Turkey. Clinical data, survival outcome of sixty patients who were diagnosed with WAIHA were retrospectively analyzed. All the patients were direct antiglobulin test (DAT) positive. In 21 (30%) patients, IgG plus C3d DAT positivity was documented. 16 patients were secondary WAIHA and most common underlying causes were lymphoproliferative diseases (5 patients) and connective tissue disease (8 patients). Corticosteroids were first choice as a first line therapy with 54.5% CR and 40.2% PR rates. 43.3% of the patients relapsed after a median 12 months. In relapsed patients, rituximab and splenectomy achieved 85% overall response rates. The median OS was not reached. The median DFS was 40 months (95% CI, 19.6–60.4). OS and DFS at 36 months were 89.6% and 51.1%, respectively. DFS at 36 months was lower in patients with IgG plus C3d positive DAT than patients with only positive Ig G DAT (36 vs. 54%) but this difference could not reach statistical significance (p = 0.23). WAIHA was a rare disease with a good prognosis. Corticosteroids were the first option and splenectomy and rituximab received good responses in relapsed patients. Attention should be paid especially in patients with IgG plus C3d DAT positivity since lower DFS were reported. Characteristics and pathogenesis of patients with IgG plus C3d DAT positivity was still an obscure.

Thrombotic microangiopathy(TMA) is a pathological diagnosis characterized by abnormalities of small vessels leading to microvascular thrombosis of arterioles and capillaries. The current prospective, non-interventional, multicenter (n:18) study aimed to define distribution of different TMA forms in adult Turkish patients who were referred for therapeutic plasma exchange (TPE) for a presumptive diagnosis of TMA. Patients with serum ADAMTS13 activity 10 %, normal renal function and no secondary TMA were treated as unclassified TMA. The study included a total of 97 patients (female: 60; male: 30) with a median age of 48 (18?74). Detailed evaluation at 1 month after hospital admission revealed aTTP, secondary TMA, infection/complement-associated hemolytic uremic syndrome and unclassified TMA in 32 (33 %), 33 (34 %), 26 (27 %) and 6 (6%) patients respectively. As subclassification of various TMAs will dictate specific therapy, proper diagnosis in a timely manner is of utmost clinical significance. © 2021 Elsevier Ltd, Alexion Pharmaceuticals: 100064, The present study was designed as an investigator initiated trial (IIT) and sponsored by Alexion Pharmaceuticals (Tracking number: 100064)

Objective: The aim of this study was to evaluate the effectiveness of cryotherapy on the prevention of oral mucositis (OM) and on the oral pH value in patients with multiple myeloma undergoing autologous stem cell transplantation. Data Sources: This nonrandomized controlled clinical trial was carried out in Bone Marrow Transplant Centers of three hospitals with total 32 patients. In addition to standard oral care, a total of 80 minutes of cryotherapy was applied to the experimental group. OM was assessed according to the World Health Organization's Oral Toxicity Scale before chemotherapy and for 21 days after chemotherapy (every day in the first 14 days, then every other day until the 21st day [if not discharged]). Conclusion: According to the findings, cryotherapy did not change the incidence of oral OM, and neither affected the severity of nor decreased the duration of it. Oral pH value was found to be significantly different between the patient groups only before and 1 day after chemotherapy. Implications for Nursing Practice: Cryotherapy is an inexpensive, easy-to-use method with no side effects; it would be beneficial to continue cryotherapy to prevent the development of OM in patients with cancer receiving drugs with a short half-life such as melphalan. It is also recommended to conduct further studies with different chemotherapy drugs with short half-lives to determine its effect on the prevention of OM development. (c) 2021 Elsevier Inc. All rights reserved.

Introduction The secretion of monoclonal immunoglobulins increase in chronic inflammatory disorders such as chronic infections and autoimmune diseases. This risk is further increased by the biological agents used in the treatment of autoimmune diseases such as psoriasis. Hematological malignancies occurring in patients with psoriasis provides an opportunity to evaluate the effect of autologous or allogeneic stem cell transplantation in this immune-mediated disease. Cases Four patients diagnosed with psoriasis are presented, having undergone autologous bone marrow transplantation (ABMT), and eventually having remission of their psoriasis, after developing multiple myeloma during follow up. Psoriasis history of the patients was 20, 23, 2 and 2 years, respectively. All of them received peroral methotrexate or topical corticosteroid therapy. Time until myeloma diagnosis were 220, 144, 25, 18 months and follow-up after ABMT were 26, 19, 15, 22 months, respectively Conclusion Psoriasis can be effectively treated with stem cell transplantation that is used in the treatment of malignancies. For this reason, stem cell transplantation can be considered as a treatment option in these patients, considering the benefit-to-harm ratio. However, uncertainty continues regarding the autologous or allogeneic application of stem cell transplantation

Introduction Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy, which develops as a result of defective activity of the alternative complement pathway and excessive complement activation due to genetic or acquired factors. No satisfactory responses were obtained by plasmapheresis, corticosteroids and fresh frozen plasma (FFP) transfusion. However, promising results are obtained in recent years by eculuzimab treatment, which inhibits C5 activation. Objective To evaluate the efficacy, safety and effect of eculizumab on quality of life of adult aHUS patients followed in our center. Materials and Methods Seven patients who received eculizumab treatment in single center between the years 2012 and 2016 due to aHUS diagnosis were retrospectively evaluated. Patients were diagnosed with aHUS in accordance with certain criteria, after eliminating all the other factors caused by thrombotic microangiopathy. Complement gene mutations were completed in six patients. All patients received eculizumab as recommended (900 mg/per two weeks) following plasmapheresis, FFP, corticosteroid and hemodialysis (HD) treatments. Results Four out of seven patients were men and three were women; average patient age was 51.1 (26–69) years and average duration of disease was 25.3 (2–45) months. Average period from the initial complaints of the patients up to aHUS diagnosis was 4.2 (2–13) months. Tests were implemented on six patients for complement gene mutations, and complement factor H (CFH) homozygous mutation was identified in three patients. Complete remission was observed in four patients and partial remission in two patients after eculizumab; however, one patient died. Plasmapheresis was discontinued in patients with complete remission, whereas two patients with partial remission continued the HD program, despite normalization in hematologic parameters. Significant improvement was observed in post-treatment quality of life in all six patients who currently continue eculuzimab treatment. No transfusion reaction and/or no serious infections were observed in any of the patients, while URTI (upper respiratory tract infection) was observed in one patient. Discussion Eculizumab is an effective and safety treatment option in adult aHUS patients. Early diagnosis and initializing eculizumab therapy at an early stage may decrease mortality and morbidity in patients with aHUS. New studies are required on this topic.

Background: Detailed history taking, physical examination and laboratory tests are useful tools to document any abnormal bleeding risk before an operation or an invasive procedure. Although coagulation tests are routinely used to demonstrate the pathological situations at the coagulation cascade or to follow-up the anticoagulation therapies, their role in determining the bleeding risk in preoperative patients is controversial. Materials and Methods: In this study, we aimed to evaluate the patients referring to our hematology clinic at Izmir Katip Celebi University Hospital for preoperative consultation due to elevated levels of coagulation tests. Results: Fifty-six patients with high PT/PTT levels were enrolled in this study. Twenty-six (46.4%) patients were male and 30 (53.6%) were female. The median age was 34 (18-75) years. We documented bleeding history in 12 (21.4%) patients. The patients having a bleeding history revealed mostly abnormal uterine bleeding, epistaxis, and gingival bleeding. Life threatening bleeding was not reported in any of the patients. The operations were cancelled or postponed at least one month in 38 (67.8%) and 10 (17.8%) patients, respectively. Per-operative or post-operative abnormal bleeding was not documented. We did not find any statistically significant difference between groups with or without elevated coagulation tests in terms of abnormal bleeding in the operations. Conclusion: Coagulations tests should be studied in selected group of patients. Additionally, mildly elevated results should be interpreted carefully to decrease the rate of cancellation and delay in operations and unnecessary increase in costs.

Immune checkpoint inhibitors are revolutionized drugs for cancer immunotherapy in the last years. The mechanism of action of CPIs including the limitation of the activation of Tcells, and thus enhancing the self-immune response against tumour cells. Checkpointinhibitors( CPIs) may dysregulate the immune system, resulting in some toxicities. These toxicities or side effects are called Immune-related Adverse Events (IRAEs) that can potentially affect any organ and tissue. Rheumatic diseases due to checkpoint inhibitors are also reported in the literature. The spectrum of rheumatic manifestations are quite wide; the most common are arthralgia/arthritis, myalgia/myositis, polimyalgia rheumatica, lupus, rheumatoid arthritis, Sjögren’s syndrome. At the same time, these drugs can also cause an exacerbation of known rheumatologic disease. Treatment approaches for developing rheumatic findings due to checkpoint inhibitors should be multidisciplinary. There should be a close relationship between oncologists who follow-up these patients and rheumatologists. The rheumatic manifestations should be defined and treated early. In general, the musculoskeletal side effects are transient and may regress after stopping CPIs. The most commonly used medications are corticosteroids. Immunosuppressive drugs (HQ, MTX, anti-TNF-alpha, anti-IL-6) should be preferred when treatment is unresponsive or as steroid-sparing agents. : The aim of this review was to evaluate the checkpoint inhibitors-related rheumatologic findings and therapeutic strategies in light of recent literature data.

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease which diagnosis may be delayed due to variable clinical findings. We describe herein a case of PNH in a 21 year old woman who admitted with complaints of chronic weakness, intermittent spontaneous ecchymoses, and an intermittent abdominal pain. On laboratory tests thrombocytopenia and iron deficiency anemia without any clinical findings were found. Flow cytometric evaluations showed a PNH clone of 15% for erythrocytes, 64% for monocytes, and 60% for granulocytes. The patient was diagnosed with PNH and an eculizumab therapy was initiated. Following initiation of eculizumab therapy, the frequency of abdominal pain attacks decreased, hemoglobin level normalized, and platelet values increased slightly. In patients submitting with a triad of symptoms such as thrombocytopenia, iron deficiency anemia, and abdominal pain attacks of unknown etiology we suggest considering PNH. We also encourage physicians to share their similar observations in order to raise the knowledge on infrequent presentations of PNH. © F. Gediz et al., 2017.